American College of Rheumatology
Scleroderma
What is Scleroderma?
Scleroderma is a chronic autoimmune disease which was first described in the 18th century. The term scleroderma means "hard skin," which describes thickening of the skin from increased deposition of coliagen.
There are two types of scleroderma. Localized scleroderma affects the skin and musculoskeletal system. Systemic sclerosis causes internal organ damage including scarring and blood vessel damage in the skin, lungs, heart, and kidneys. It can slow contractions in the gastrointestinal tract and cause arthritis. Systemic sclerosis can also produce muscle inflammation, dry eyes, and dry mouth. Most people with scleroderma have cold-induced spasms of small blood vessels in their hands or feet, known as Raynaud's phenomenon. Systemic sclerosis with widespread skin involvement more often results in severe internal organ damage than in limited skin disease.
Cause
In most cases, the cause of scleroderma is unknown. However, in a small minority of cases, scleroderma or scleroderma-like illnesses are associated with exposure to certain toxins or as a complication of bone marrow transplantation. Scleroderma is not contagious and is rarely inherited.
Systemic sclerosis is associated with activation of cells in the immune system, increased production of scar tissue by cells called fibroblasts within the skin and internal organs, and damage to endothelial cells that line small blood vessels leading to the over-production of scar tissue.
Health Impact
* Affects women more than men and adults more than children
* 10-20 new cases per million people each year
* 60 percent to 70 percent five-year survival rate
* Lung,heart,and kidney damage are the most frequent causes of severe disability and death
* Many people have decreased hand function because of joint disfigurement or finger ulcers
Diagnosis
Diagnosis for scleroderma is based on clinical history and physical findings. Diagnosis may be delayed in those without significant skin thickening. Laboratory, radiological, and pulmonary function tests determine the extent and severity of internal organ involvement.
Treatment
There is no known cure for scleroderma. No treatment has been scientifically proven to alter the overall course of the disease, although d-penicillamine is commonly used for this purpose and may be of some value. There are a number of effective organ-specific treatments for scleroderma. Raynaud’s phenomenon may be helped by calcium channel blockers. Declining renal function and hypertension are often treated with drugs. Esophageal damage from reflux of stomach contents can be treated with acid-reducing drugs. Antibiotics, special diets, and medication can improve absorption of nutrients in people who have contractions of their intestines. Musculoskeletal pain may respond to nonsteroidal anti-inflammatory agents.Physical and occupational therapies are used to minimize joint disability and functional impairment.
The Rheumatologist's Role in Treating Scieroderma
Rheumatologists are specially trained in caring for people with autoimmune diseases such as scleroderma. Because many different organs may be involved, the overall evaluation,and treatment of scleroderma is best managed by a rheumatologist. Rheumatologists in basic and clinical research are the leaders in improving our understanding and treatment of this rheumatic disease.
